The European Commission’s science

and knowledge service

Joint Research Centre

Coding Primary Site and Tumor
Morphology

ENCR-JRC training course

Ispra, 5-6 June 2019
Nadya Dimitrova
Outline

• What is coding and why do we need it?

• ICD-10 and ICD-O

• ICD-O-3 Topography coding rules

• ICD-O-3 Morphology coding rules

• How to code?

• ICD-O-3 online
 What is coding and why do we need it?

Coding:
• to assign numerical codes to text descriptions
Example: Malignant neoplasm of the lung = C34
Advantages:
• group cases with similar concepts – site of origin, type of cancer
• complex series of pieces of information can be:
• conveyed,
• stored and
• retrieved in the form of numbers (quickly, easier)
Cancer registry data - example
Morphology
PIN Incidence date Site and behavior grade
1011000047 04082000 C419 91803 1
1011000713 22062000 C504 85003 2
1011010324 26072001 C186 84803 2
1011010983 04052001 C56 84503 4
1011020767 30092009 C229 80003 2
1011030136 01032007 C80 80003 9
1011030898 27102005 C64 83103 4
1011040255 04012004 C629 91003 1
What is coding and why do we need it?

Coding is based on classification:

• arrangement of neoplasms or distribution in classes
according to a method or system, ensuring
comparability.

The two most important items of information are:

• Topography: the location (site) of the tumor in the
body;
• Morphology (histology, cytology): the appearance of
the tumor when examined under the microscope.
 What is ICD-O?

• Subset of International
Classification of Diseases
• Specific code set for
neoplasms
• Coding system for primary
site and cell type
• Example: adenocarcinoma
of lung = C34, 8140/3
• ICD-O-3.2 version published
in April 2019, to be used from
Original - all diseases Subset - neoplasms only
2020 - http://www.iacr.com.fr
Differences: Organization

• ICD-10 organized by
1. Behavior
2. Anatomic site involved
3. Morphology codes optional

• ICD-O organized by
1. Anatomical site involved
2. Morphology + Behavior
ICD-10 and ICD-O-3

Term ICD-10 ICD-O-3

Lung tumor, benign D14.3 C34.9 8000/0

Lung tumor, borderline D38.1 C34.9 8000/1

Lung tumor, in situ D02.2 C34.9 8000/2

Lung tumor, invasive C34.9 C34.9 8000/3

Lung tumor, metastatic C78.0 C34.9 8000/6

Lung tumor, uncertain

D38.1 C34.9 8000/9
if primary or metastatic
Differences: Codes

• Some special codes added in ICD-O

• Some ICD-10 codes not used
• Some codes used differently
 ICD-O Special Site Codes

C42 Hematopoietic and reticuloendothelial system

• C42.0 Blood
• C42.1 Bone marrow
• C42.2 Spleen
• C42.3 Reticuloendothelial system, NOS
• C42.4 Hematopoietic system, NOS

• Use as primary site for leukemia (C42.1), multiple

myeloma (C42.1), Waldenstrom macroglobulinemia
(C42.0)
ICD-10 Codes Not Used in ICD-O
Histology-specific ICD-10 Codes

ICD-10 ICD-O
C43 Melanoma of skin C44
C45 Mesothelioma C38.4*
C46 Kaposi’s sarcoma C44*
C81-C96 Lymphomas, leukemias, C42.1*, C77.*
other blood diseases
* For most cases
Other ICD-10 Codes Not Used in ICD-O

ICD-10 ICD-O
C26.1 Spleen C42.2

C78-C79 Secondary malignant neoplasm Code primary site

of other specified sites (only) in ICD-O

C97 Malignant neoplasm of Code each primary

independent multiple primary separately
sites
Site Codes Used Differently

ICD-10 ICD-O
C77 Lymph nodes Use C77._ as primary
(primary and site for nodal
secondary) lymphomas.
Do not code lymph node
metastases as primary
sites in ICD-O.
Structure of Code
Meaning
ICD-O-3 Topography Coding Rules
Coding topography using ICD-O-3

• The topography code:

• Indicates the site of origin of a neoplasm – where the
tumor arose.
• Has four-characters that run from C00.0 to C80.9
• A decimal point (.) separates subdivisions of the three-
character categories

C _ _._ C 50 . 2
site subsite breast, upper inner quadrant
ICD-O topography codes - example
C50.8
UOQ UIQ
C50.4
11 12 1C50.2
10 2
C50.0
C50.8 9 3 C50.8
C50.1
8 4
7 6 5
LOQ LIQ
C50.5 C50.3
C50.8
RIGHT BREAST
ICD-O topography codes - example
Special topography codes

Divisions of the Esophagus – two incompatible systems

and both are included.
• C15 Esophagus
• C15.0 Cervical esophagus radiographic and intraoperative

• C15.1 Thoracic esophagus descriptors

• C15.2 Abdominal esophagus

• C15.3 Upper third of esophagus endoscopic and clinical

• C15.4 Middle third of esophagus descriptors

• C15.5 Lower third of esophagus

• C15.8 Overlapping lesion of esophagus
• C15.9 Esophagus, NOS
ICD-O-3 Topography Coding Rules

• Rule A. Topographic regions and ill-defined

sites
• Rule B. Prefixes
• Rule C. Tumors involving more than one
topographic category or subcategory
• Rule D. Topography codes for lymphomas
• Rule E. Topography code for leukaemias
 Rule A
Topographic regions and ill-defined sites
Rule A
Topographic Regions and Ill-defined Sites
“If the diagnosis does not specify the tissue of origin, code the
appropriate tissues suggested in the alphabetic index for each ill-
defined site in preference to the "NOS" category.”
Ill-defined site: term for area of the body used in a general sense -
arm, ankle, face
NOS: Not otherwise specified
• Not elsewhere classified
• Term used in a general sense
• When there are more specific codes
• To encompass an organ as a whole
Rule A
Topographic Regions and Ill-defined Sites
• Example: Osteosarcoma of ankle
• Issue: Primary site not fully identified in diagnosis
• Several non-specific codes available in the alphabetic
index
• C76.5 Ankle, NOS
• C40.3 Bone of ankle
• C44.7 Melanoma of ankle
• C49.2 Soft tissue of ankle
• Use information from the cell type to code primary site.
• Osteosarcoma of ankle
• Code: C40.3 bone of ankle
Rule A
Topographic Regions and Ill-defined Sites
Implied rule:
• Code as specifically as you can with the information
you have
• Avoid using ill-defined site codes if possible.
Rule B
Prefixes
 Rule B. Prefixes

• "If a topographic site is modified by a prefix such as peri-

para-, or the like which is not specifically listed in ICD-O,
code to the appropriate ill-defined subcategory C76, unless
the type of tumour indicates origin from a particular tissue".
• Prefix terms in ICD-O index:
Term ICD-O code
Periampullary C24.1
Periadrenal or perirenal C48.0
Retrocecal or peripancreatic C48.0
Supratentorial brain C71.0
Rule B. Prefixes

• When term is not listed, determine the location it

describes and code that.
• Examples
Paraspinal—along the spine:
C76.7 Ill-defined site of back
Perigastric—near the stomach:
C76.2 Ill-defined site of abdomen
Peribiliary—near the biliary tract:
C76.2 Ill-defined site of abdomen
Rule B. Prefixes

• Implied rule: When you can’t find a code for the

anatomic site term in the alphabetic index, use “ill-
defined site of …” C76._
• Do not code to primary site mentioned (such as spine,
stomach, bile duct) when tumor is described as “near”
that organ.
 Rule C
Tumors involving more than one
topographic category or subcategory
Rule C. Tumors Involving More Than One
Topographic Category or Subcategory
• “Use subcategory ".8" when a tumor overlaps the
boundaries of two or more categories or subcategories
and its point of origin cannot be determined.”

• Implied rule: If you can’t tell where a single tumor

started in an organ or system, use an “overlapping site
code, C_ _ . 8.
Rule C. Overlapping Sites - examples

Overlapping lesion of ICD-O code

Tongue C02.8
Major salivary glands C08.8
Lip, oral cavity and pharynx C14.8
Rectum, anus and anal canal C21.8
Biliary tract C24.8
Digestive system C26.8
Female genital organs C57.8
Male genital organs C63.8
 Rule C. Overlapping Sites - examples

Large neoplasm of descending Glioma involving parietal

Tumor of breast at 12:00 – C50.8
and sigmoid colon – C18.8 and occipital lobes – C71.8
Rule C. Overlapping Sites

Does not apply to non-adjacent sites

• Example: Squamous cell carcinoma of skin of forehead
and skin of forearm
• Primary site: C44.9 Skin, NOS
Forearm and forehead are not adjacent sites.
Does not apply to separate lesions
• Example: Separate tumors in UIQ and LOQ of breast
• Primary site: C50.9 Breast, NOS (or according to the rules
for multiple primaries)
 Rule D
Topography codes for lymphomas
 Rule D. Topography Codes for Lymphomas
Lymphomas arising in lymph nodes
Code C77._
If multiple lymph node regions are
involved, code to C77.8 (lymph nodes of
multiple regions).
If no site is indicated for a [nodal]
lymphoma, code to C77.9 (lymph node,
NOS).
Extranodal lymphomas
Code to the site of origin, which may not be
the site of the biopsy.
If no site is indicated for a lymphoma and it
is suspected to be extranodal, code to C80.9
(unknown primary site).
Rule D. Topography Codes for Lymphomas

Implied rule: Code a lymphoma to its site of origin.

Examples:
• Hodgkin lymphoma of cervical lymph nodes C77.0
• MALT lymphoma of stomach C16.9
• B-cell lymphoma of inguinal and femoral nodes C77.4
• Primary lymphoma of femur C40.2
Rule D. Topography Codes for Lymphomas

C77.8 Special Rule: Involved lymph

node chains do not have to be adjacent.

Examples:
Cervical and mediastinal nodes C77.8
Axillary and periaortic nodes C77.8
 Rule E
Topography code for leukaemias
Rule E. Topography Code for Leukemias

“Code all leukaemias except myeloid sarcoma (M-9903/3)

to C42.1, bone marrow.”
• Myeloid sarcoma: a deposit of malignant myeloid cells
in soft tissue. Code to location of lesion.
• The following are also coded to bone marrow, C42.1
• Multiple myeloma
• Refractory anemias
• Polycythemia vera
• Myelodysplastic syndrome
• Other hematopoietic diseases
ICD-O-3 Morphology Coding Rules
ICD-O-3 Morphology Coding Rules

• Rule F. Behavior code in morphology

• Rule G. Grading or differentiation code
• Rule H. Site-associated morphology terms
• Rule J. Compound morphology diagnoses
• Rule K. Coding multiple morphology terms

Note: ICD-O-3.2 includes about 100 new terms, some of

them with new codes
 Rule F
Behavior code in morphology
Rule F. Behavior Code in Morphology

• “Use the appropriate 5th digit behavior code even if the

exact term is not listed in ICD-O.”

___ ___ ___ ___ / ___ ___

Cell type
Behavior
Differentiation
 Rule F. Behavior Code in Morphology
• Behavior of a tumor is the way it acts within the body
• Codes:
• /0 – benign – the tumor grows in place without the potential for
spread;
• /1 – uncertain whether benign or malignant
• /2 – noninvasive or in situ – the tumor is malignant, but still growing
in place
• /3 – malignant, primary site – the tumor invades surrounding tissues
• /6* – malignant, metastatic site – the tumor disseminates from its
point of origin and begin to grow at another site
• /9* – malignant, uncertain whether primary or metastatic site
• * Not used by cancer registries, but by pathologists
Rule F. Behavior Code in Morphology

• The Matrix principle

8010/0 Epithelial tumor, benign
8010/1* Borderline epithelial tumor
8010/2 Intraepithelial carcinoma, NOS
8010/3 Epithelial tumor, malignant (Carcinoma, NOS)
8010/6 Metastatic carcinoma
8010/9 Carcinomatosis

* 8010/1 is not printed in ICD-O-3, but can be constructed if the

pathologist diagnoses a borderline epithelial tumor.
 Rule F. Behavior Code in Morphology

Implied rule: It is OK to change the behavior code to

accurately report what the pathologist said.

Example:
• Pathology report states “adenoid squamous cell carcinoma in situ”
ICD-O-3 only lists 8075/3 Adenoid squamous cell carcinoma.
Change behavior code to 8075/2 to indicate in situ.
 Rule F. Behavior Code in Morphology

• ICD-O-3.2
• About 65 changes (or new) of behavior code
• May impact on the definition of a reportable case
• Examples:
Change of behavior code (from 1) 8150 3 Pancreatic endocrine tumor, NOS (C25.4)
Change of behavior code (from 1) 8580 3 Thymoma, NOS (C37.9)
Change of code and behavior
8500 2 Cystic hypersecretory carcinoma (C50._)
(was 8508/3)

Change of behavior code (from 3) 8832 1 Dermatofibrosarcoma, NOS (C44._)

 Rule F. Behavior Code in Morphology

Note: Most cancer registries collect only

/2 Carcinoma in situ; noninvasive; noninfiltrating; intraepithelial
/3 Malignant, primary site (invasive)
• If diagnosis comes from a metastatic site, the cancer registry
records primary site and morphology with behavior /3.
Example: Pathology report says: “metastatic adenocarcinoma in
lung biopsy.” Coded as C34.9 8140/6 on report.
Cancer registry reports case as unknown primary site, C80.9
8140/3
 Rule G
Grading or differentiation code
Rule G. Grading or Differentiation Code

• "Assign the highest grade or differentiation code

described in the diagnostic statement."

___ ___ ___ ___ / ___ ___

Cell type
Behavior
Differentiation
 Rule G. Grading or Differentiation Code

Histologic grading and differentiation for malignant

tumors – describes how much or how little a tumor
resembles the normal tissue from which it arose (codes 1 to
4 and 9).
Codes:
1 – Grade I, well differentiated
2 – Grade II, moderately differentiated
3 – Grade III, poorly differentiated
4 – Grade IV, undifferentiated, anaplastic
9 – grade, differentiation or cell type not determined, not
stated or not applicable
 Rule G. Grading or Differentiation Code

• Cell origin/lineage for leukemias and lymphomas (codes

5 to 8 and 9)
Codes:
5 – T-cell
6 – B-cell
7 – Null cell, Non T-non B
8 – NK cell
9 – grade, differentiation or cell type not determined, not
stated or not applicable
Rule G. Grading or Differentiation Code

Implied rule: Code to the higher grade.

Example: moderately to poorly differentiated
adenocarcinoma of prostate

• Moderately differentiated = grade 2

• Poorly differentiated = grade 3
• Code diagnosis as 8140/33
Rule G. Grading or Differentiation Code

• Implied rule: “double code” any statement of grade in

the diagnostic term.

Example: undifferentiated carcinoma

• Undifferentiated carcinoma= 8020/3
• Undifferentiated = 4
• Code diagnosis as 8020/34
 Rule G. Grading or Differentiation Code

Central Nervous System tumours follow a slightly different

system (table 27 in ICD-O)
WHO Grade I (benign or borderline malignant)
WHO Grade II (‘low grade’)
WHO Grade III (‘anaplastic’)
WHO Grade IV
Examples:
oligodendroglioma, NOS 9450/32
anaplastic astrocytoma 9401/33
glioblastoma 9440/34
 Rule G. Grading or Differentiation Code

Implied rule: For haematological malignancies use cell origin, not

the differentiation (grade), if both are stated.

Example:
• Poorly differentiated B-cell nodular lymphocytic lymphoma
Poorly differentiated = grade 3
B-cell origin = code 6
Code diagnosis as 9693/36
 Rule H
Site-associated Morphology Terms
 Rule H. Site-associated Morphology Terms

“Use the topography code provided when a topographic site is not stated
in the diagnosis.
This topography code should be disregarded if the tumor is known to
arise at another site.”
Suggested site code
• In parenthesis ( ) after morphology term
• Most common site associated with neoplasm
Examples:
M-8330/3 Adenocarcinoma, follicular (C73.9 {thyroid})
M-9700/3 Mycosis fungoides (C44._ {skin})
 Rule H. Site-associated Morphology Terms

Examples of terms that include a root word mentioning a site

• Nephroblastoma, NOS (C64.9 – kidney)
• Thymoma, NOS (C37.9 – thymus)
• Bronchiolar carcinoma (C34._ – bronchioles of lung)
• Hepatocellular carcinoma (C22.0 – liver)
No suggested site code is listed when malignancy could appear
in many sites, such as adenocarcinoma, NOS
 Rule H. Site-associated Morphology Terms

If a site is given that is different from the site indicated by the

suggested site code, use the site code appropriate to the diagnosis.
Example: infiltrating duct carcinoma, head of pancreas
• 8500/3 Infiltrating duct carcinoma (C50._) Suggested site code is
breast—ignore this based on diagnosis!
• Head of pancreas = C25.0
• Code diagnosis as C25.0 8500/3

Implied rule: Use the suggested site code as a guide to code the
primary site. If the site stated in the pathology report is different, code
what the pathology report states (note: metastatic site).
 Rule J
Compound Morphology Diagnoses
Rule J. Compound Morphology Diagnoses

“Change the order of word roots in a compound term if

the term is not listed in ICD-O-3.”
Rule J. Compound Morphology Diagnoses

Compound terms have multiple root words

Example:
• Myxofibrosarcoma – not in ICD-O-3.1 (added to ICD-O-3.2)
Break into word roots
Myxo / fibro / sarcoma
Change around word roots, then look up new term
Fibromyxosarcoma 8811/3
• Chondro-osteosarcoma
Osteochondrosarcoma 9180/3
 Rule K
Coding Multiple Morphology Terms
 Rule K. Coding Multiple Morphology Terms

“When no single code includes all diagnostic terms, use the numerically
higher code number if the diagnosis of a single tumor includes two
modifying adjectives with different code numbers.”

Implied rule: Code to higher code number if there is no combination

code.
For haematological malignancies - code the most specific code (not
necessarily the highest code), take into account specific diagnostics, such
as immunohistochemistry and cytogenetics.
 Rule K. Coding Multiple Morphology Terms

Examples:
• “Adenoid cystic spindle cell carcinoma”
No combination code
Spindle cell carcinoma 8032/3
Adenoid cystic carcinoma 8200/3
Code morphology to higher number 8200/3
• “Papillary and tubular adenocarcinoma”
Papillary adenocarcinoma 8260/3
Tubular adenocarcinoma 8211/3
There is a singe code: 8255/3 (Adenocarcinoma with mixed
subtypes)
 Rule K. Coding Multiple Morphology Terms

Look for a code that represents the combined morphology:

Mixed embryonal carcinoma and teratoma = teratocarcinoma 9081/3
Ductal carcinoma and lobular carcinoma 8522/3
• Compound terms
Carcinosarcoma 8980/3
Adenocarcinoma and squamous carcinoma = adenosquamous
carcinoma 8560/3
Small cell-large cell carcinoma 8045/3
How to Code

• Break phrase into topography and morphology

• Look up morphology first
• Use up all the words in the phrase
• Add 5th and 6th digit codes
• Look up topography
 Coding example 1

Diagnosis: Poorly differentiated hepato-cellular carcinoma of

right lobe of liver
• What is it (morphology and behavior)?
Hepatocellular carcinoma, NOS (C22.0): 8170/3
• Suggested site code is included in index
Liver, right lobe: C22.0 Liver, NOS
• What else do we know?
Poorly differentiated: /_3
• Complete codes: C22.0 8170/33
 Coding example 2

Diagnosis: Moderately differentiated adenocarcinoma of

prostate
• What is it (morphology and behaviour)?
Adenocarcinoma [not otherwise specified]: 8140/3
• What else do we know?
Moderately differentiated: /_2
• Where did it start (topography)?
Prostate: C61.9
• Complete codes: C61.9 8140/32
ICD-O-3 online: codes.iarc.fr